Congenital cholesteatoma.Congenital cholesteatomas are benign tumors of the temporal bone that originate in the middle ear. They are believed to occur secondary to a failure of the normal involution involution /in·vo·lu·tion/ (in?vo-loo´shun) 1. a rolling or turning inward. 2. a retrograde change of the body or of an organ, as the retrograde changes in size of the female genital organs after delivery. of embryonic epidermoid epidermoid /epi·der·moid/ (-der´moid) 1. pertaining to or resembling the epidermis. 2. epidermoid cyst. ep·i·der·moid adj. Composed of or resembling epidermal tissue. tissue. (1) These lesions classically present as a white mass behind an intact tympanic membrane, typically in the anterosuperior quadrant. In the accompanying photograph, a congenital cholesteatoma is seen in the posterior quadrant (figure). [FIGURE OMITTED] When a congenital cholesteatoma in the anterosuperior quadrant is diagnosed early, complete surgical removal is generally successful. Post-operative function is usually excellent because these cysts are well encapsulated and do not affect the ossicular os·si·cle n. A small bone, especially one of the three bones of the middle ear. [Latin ossiculum, diminutive of os, bone; see ost- in Indo-European roots. chain. But once a tumor grows to involve the posterosuperior quadrant, or when one originates in the posterior quadrant, involvement of the ossicular chain is usually the rule. Removal of such a cholesteatoma may require sacrificing the malleus malleus /mal·le·us/ (mal´e-us) [L.] the outermost of the auditory ossicles, and the one attached to the tympanic membrane; its club-shaped head articulates with the incus mal·le·us n. pl. and incus incus /in·cus/ (ing´kus) [L.] the middle of the three ossicles of the ear, which, with the stapes and malleus, serves to conduct vibrations from the tympanic membrane to the inner ear. Called also Congenital cholesteatomas that occupy the anterosuperior aspect of the middle ear are asymptomatic and are usually found on routine otoscopic examination. In some cases, the initial diagnosis is made in the operating room during the placement of a myringotomy myringotomy /my·rin·got·o·my/ (mi-ring-got´ah-me) tympanotomy; creation of a hole in the tympanic membrane, as for tympanocentesis. myr·in·got·o·my n. tube; an enlarging cholesteatoma will cause tympanic membrane perforation tympanic membrane perforation Perforated, punctured, ruptured ear drum ENT A disruption of the tympanic membrane due to acoustic trauma, direct injury, barotrauma, introduction of Q-tips or small objects, or infection with fluid buildup in the middle ear. See Tympanoplasty. and chronic or intermittent otorrhea. When diagnosis is delayed to this point, a congenital cholesteatoma cannot be distinguished with certainty from one that is acquired. Treatment of congenital cholesteatomas entails surgical excision. Children with extensive disease may require a second-stage procedure to look for regrowth of microscopic residual disease and to reconstruct the ossicular chain. The second procedure usually is performed 9 to 12 months after the initial surgery. Long-term follow-up is critical in these cases. (2) References (1.) Levenson M J, Michaels L, Parisier SC. Juarbe C Congenital cholesteatomas in children: An embryologic correlation. Laryngoscope 1988;98:949-55. (2.) Parisier SC, Levenson M J, Edelstein DR, et al. Management of congenital pediatric cholesteatomas. Am J Otol 1989;10:121-3. |
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